Child's Nervous System

, Volume 23, Issue 4, pp 399–405

Outcome of children with posterior fossa medulloblastoma: a single institution experience over the decade 1994–2003

  • D. Kombogiorgas
  • S. Sgouros
  • A. R. Walsh
  • A. D. Hockley
  • M. Stevens
  • R. Grundy
  • A. Peet
  • M. English
  • D. Spooner
Original Paper

Abstract

Aim

While the impact of radiotherapy in the management of medulloblastoma was recognised, the introduction of chemotherapy was investigated in clinical trials and shown to confer an additional advantage. We reviewed the outcome of a series of consecutive patients to assess the impact in a population-based clinical establishment.

Materials and methods

A series of 38 children treated for medulloblastoma at Birmingham Children’s Hospital between 1994 and 2003 was analysed. The effect of surgery, radiotherapy, chemotherapy and metastasis on survival was analysed.

Results

The overall 5-year survival rate was 61.4% for the 36 patients who had resective surgery, while 2 patients had biopsy only and died within a few months. There was no operative mortality. The incidence of hydrocephalus needing permanent shunting was higher in the first 3 years of life (p = 0.007, chi-square). The 5-year survival rate of patients with total and sub-total excision of medulloblastoma was 61.1% and 61.8%, respectively. The 5-year survival rate of patients older than 3 years was 73.4% and for patients under 3 years was 36.3% (p = 0.007, log rank). Metastases at presentation did not influence survival. All deaths occurred in the first 32 months.

Conclusion

The contribution of chemotherapy in the improvement of the overall survival appears more evident in children younger than 3 years or presenting with metastases. The absence of significant difference in survival between patients with total or sub-total excision of medulloblastoma supports the view that total excision of medulloblastoma can be avoided when the risk for potential intra-operative damage and consequent neurological deficits is high.

Keywords

Medulloblastoma Surgery Radiotherapy Chemotherapy 

References

  1. 1.
    Albright AL, Wisoff JH, Zeltzer PM, Boyett JM, Rorke LB, Stanley P (1996) Effects of medulloblastoma resections on outcome in children: a report from the Children’s Cancer Group. Neurosurgery 38(2):265–271PubMedCrossRefGoogle Scholar
  2. 2.
    Calaminus G, Janssen G, Lenard HG, Bock WJ, Reifenberger G, Schmitt G, Gobel U (1998) Combined therapy of medulloblastoma: review of 46 patients treated in a single institution. Neuropediatrics 29(2):102–107PubMedGoogle Scholar
  3. 3.
    Carrie C, Hoffstetter S, Gomez F, Moncho V, Doz F, Alapetite C, Murraciole X, Maire JP, Benhassel M, Chapet S, Quetin P, Kolodie H, Lagrange JL, Cuillere JC, Habrand JL (1999) Impact of targeting deviations on outcome in medulloblastoma: study of the French Society of Pediatric Oncology (SFOP). Int J Radiat Oncol Biol Phys 45(2):435–439PubMedCrossRefGoogle Scholar
  4. 4.
    Carrie C, Muracciole X, Gomez F, Habrand JL, Benhassel M, Mege M, Mahe M, Quetin P, Maire JP, Soum F, Baron MH, Clavere P, Chapet S, Gaci Z, Kolodie H, Maingon P, Vie B, Bernier V, Alapetite C, Hoffstetter S, Grill J, Lafay F, French Society of Pediatric Oncology (2005) Conformal radiotherapy, reduced boost volume, hyperfractionated radiotherapy, and online quality control in standard-risk medulloblastoma without chemotherapy: results of the French M-SFOP 98 protocol. Int J Radiat Oncol Biol Phys 63(3):711–716PubMedCrossRefGoogle Scholar
  5. 5.
    David KM, Casey AT, Hayward RD, Harkness WF, Phipps K, Wade AM (1997) Medulloblastoma: is the 5-year survival rate improving? A review of 80 cases from a single institution. J Neurosurg 86(1):13–21PubMedCrossRefGoogle Scholar
  6. 6.
    Deutsch M (1988) Medulloblastoma: staging and treatment outcome. Int J Radiat Oncol Biol Phys 14(6):1103–1107PubMedGoogle Scholar
  7. 7.
    Donahue B (1992) Short- and long-term complications of radiation therapy for pediatric brain tumors. Pediatr Neurosurg 18(4):207–217PubMedGoogle Scholar
  8. 8.
    Eberhart CG, Kepner JL, Goldthwaite PT, Kun LE, Duffner PK, Friedman HS, Strother DR, Burger PC (2002) Histopathologic grading of medulloblastomas: a Pediatric Oncology Group study. Cancer 94(2):552–560PubMedCrossRefGoogle Scholar
  9. 9.
    Ellison DW, Clifford SC, Gajjar A, Gilbertson RJ (2003) What’s new in neuro-oncology? Recent advances in medulloblastoma. Eur J Paediatr Neurol 7(2):53–66PubMedCrossRefGoogle Scholar
  10. 10.
    Evans AE, Jenkin RD, Sposto R, Ortega JA, Wilson CB, Wara W, Ertel IJ, Kramer S, Chang CH, Leikin SL et al (1990) The treatment of medulloblastoma. Results of a prospective randomized trial of radiation therapy with and without CCNU, vincristine, and prednisone. J Neurosurg 72(4):572–582PubMedGoogle Scholar
  11. 11.
    Freeman CR, Taylor RE, Kortmann RD, Carrie C (2002) Radiotherapy for medulloblastoma in children: a perspective on current international clinical research efforts. Med Pediatr Oncol 39(2):99–108PubMedCrossRefGoogle Scholar
  12. 12.
    Gajjar A, Sanford RA, Bhargava R, Heideman R, Walter A, Li Y, Langston JW, Jenkins JJ, Muhlbauer M, Boyett J, Kun LE (1996) Medulloblastoma with brain stem involvement: the impact of gross total resection on outcome. Pediatr Neurosurg 25(4):182–187PubMedGoogle Scholar
  13. 13.
    Geyer JR, Sposto R, Jennings M, Boyett JM, Axtell RA, Breiger D, Broxson E, Donahue B, Finlay JL, Goldwein JW, Heier LA, Johnson D, Mazewski C, Miller DC, Packer R, Puccetti D, Radcliffe J, Tao ML, Shiminski-Maher T, Children’s Cancer Group (2005) Multiagent chemotherapy and deferred radiotherapy in infants with malignant brain tumors: a report from the Children’s Cancer Group. J Clin Oncol 23(30):7621–7631PubMedCrossRefGoogle Scholar
  14. 14.
    Gilbertson RJ (2004) Medulloblastoma: signalling a change in treatment. Lancet Oncol 5(4):209–218PubMedCrossRefGoogle Scholar
  15. 15.
    Giordana MT, Schiffer P, Schiffer D (1998) Prognostic factors in medulloblastoma. Childs Nerv Syst 14(6):256–262PubMedCrossRefGoogle Scholar
  16. 16.
    Grill J, Sainte-Rose C, Jouvet A, Gentet JC, Lejars O, Frappaz D, Doz F, Rialland X, Pichon F, Bertozzi AI, Chastagner P, Couanet D, Habrand JL, Raquin MA, Le Deley MC, Kalifa C, French Society of Paediatric Oncology (2005) Treatment of medulloblastoma with postoperative chemotherapy alone: an SFOP prospective trial in young children. Lancet Oncol 6(8):573–580PubMedCrossRefGoogle Scholar
  17. 17.
    Halperin EC, Friedman HS (1996) Is there a correlation between duration of presenting symptoms and stage of medulloblastoma at the time of diagnosis? Cancer 78(4):874–880PubMedCrossRefGoogle Scholar
  18. 18.
    Jenkin D, Goddard K, Armstrong D, Becker L, Berry M, Chan H, Doherty M, Greenberg M, Hendrick B, Hoffman H et al (1990) Posterior fossa medulloblastoma in childhood: treatment results and a proposal for a new staging system. Int J Radiat Oncol Biol Phys 19(2):265–274PubMedGoogle Scholar
  19. 19.
    Johnson DL, McCabe MA, Nicholson HS, Joseph AL, Getson PR, Byrne J, Brasseux C, Packer RJ, Reaman G (1994) Quality of long-term survival in young children with medulloblastoma. J Neurosurg 80(6):1004–1010PubMedCrossRefGoogle Scholar
  20. 20.
    Khafaga Y, Kandil AE, Jamshed A, Hassounah M, DeVol E, Gray AJ (1996) Treatment results for 149 medulloblastoma patients from one institution. Int J Radiat Oncol Biol Phys 35(3):501–506PubMedCrossRefGoogle Scholar
  21. 21.
    Kleihues P, Louis DN, Scheithauer BW, Rorke LB, Reifenberger G, Burger PC, Cavenee WK (2002) The WHO classification of tumours of the nervous system. J Neuropathol Exp Neurol 61(3):215–225PubMedGoogle Scholar
  22. 22.
    Packer RJ, Sutton LN, Goldwein JW, Perilongo G, Bunin G, Ryan J, Cohen BH, D’Angio G, Kramer ED, Zimmerman RA et al (1991) Improved survival with the use of adjuvant chemotherapy in the treatment of medulloblastoma. J Neurosurg 74(3):433–440PubMedGoogle Scholar
  23. 23.
    Packer RJ, Sutton LN, Elterman R, Lange B, Goldwein J, Nicholson HS, Mulne L, Boyett J, D’Angio G, Wechsler-Jentzsch K et al (1994) Outcome for children with medulloblastoma treated with radiation and cisplatin, CCNU, and vincristine chemotherapy. J Neurosurg 81(5):690–698PubMedGoogle Scholar
  24. 24.
    Packer RJ, Goldwein J, Nicholson HS, Vezina LG, Allen JC, Ris MD, Muraszko K, Rorke LB, Wara WM, Cohen BH, Boyett JM (1999) Treatment of children with medulloblastomas with reduced-dose craniospinal radiation therapy and adjuvant chemotherapy: a Children’s Cancer Group Study. J Clin Oncol 17(7):2127–2136PubMedGoogle Scholar
  25. 25.
    Palmer SL, Goloubeva O, Reddick WE, Glass JO, Gajjar A, Kun L, Merchant TE, Mulhern RK (2001) Patterns of intellectual development among survivors of pediatric medulloblastoma: a longitudinal analysis. J Clin Oncol 19(8):2302–2308PubMedGoogle Scholar
  26. 26.
    Paterson E, Farr RF (1953) Cerebellar medulloblastoma: treatment by irradiation of the whole central nervous system. Acta Radiol 39(4):323–336PubMedCrossRefGoogle Scholar
  27. 27.
    Perez-Martinez A, Lassaletta A, Gonzalez-Vicent M, Sevilla J, Diaz MA, Madero L (2005) High-dose chemotherapy with autologous stem cell rescue for children with high risk and recurrent medulloblastoma and supratentorial primitive neuroectodermal tumors. J Neurooncol 71(1):33–38PubMedCrossRefGoogle Scholar
  28. 28.
    Ris MD, Packer R, Goldwein J, Jones-Wallace D, Boyett JM (2001) Intellectual outcome after reduced-dose radiation therapy plus adjuvant chemotherapy for medulloblastoma: a Children’s Cancer Group study. J Clin Oncol 19(15):3470–3476PubMedGoogle Scholar
  29. 29.
    Roberts RO, Lynch CF, Jones MP, Hart MN (1991) Medulloblastoma: a population-based study of 532 cases. J Neuropathol Exp Neurol 50(2):134–144PubMedGoogle Scholar
  30. 30.
    Rutkowski S, Bode U, Deinlein F, Ottensmeier H, Warmuth-Metz M, Soerensen N, Graf N, Emser A, Pietsch T, Wolff JE, Kortmann RD, Kuehl J (2005) Treatment of early childhood medulloblastoma by postoperative chemotherapy alone. N Engl J Med 352(10):978–986PubMedCrossRefGoogle Scholar
  31. 31.
    Strother D, Ashley D, Kellie SJ, Patel A, Jones-Wallace D, Thompson S, Heideman R, Benaim E, Krance R, Bowman L, Gajjar A (2001) Feasibility of four consecutive high-dose chemotherapy cycles with stem-cell rescue for patients with newly diagnosed medulloblastoma or supratentorial primitive neuroectodermal tumor after craniospinal radiotherapy: results of a collaborative study. J Clin Oncol 19(10):2696–2704PubMedGoogle Scholar
  32. 32.
    Sutton LN, Phillips PC, Molloy PT (1996) Surgical management of medulloblastoma. J Neurooncol 29(1):9–21PubMedCrossRefGoogle Scholar
  33. 33.
    Tabori U, Sung L, Hukin J, Laperriere N, Crooks B, Carret AS, Silva M, Odame I, Mpofu C, Strother D, Wilson B, Samson Y, Bouffet E (2006) Distinctive clinical course and pattern of relapse in adolescents with medulloblastoma. Int J Radiat Oncol Biol Phys 64(2):402–407PubMedCrossRefGoogle Scholar
  34. 34.
    Tait DM, Thornton-Jones H, Bloom HJ, Lemerle J, Morris-Jones P (1990) Adjuvant chemotherapy for medulloblastoma: the first multi-centre control trial of the International Society of Paediatric Oncology (SIOP I). Eur J Cancer 26(4):464–469PubMedGoogle Scholar
  35. 35.
    Taylor RE, Bailey CC, Robinson K, Weston CL, Ellison D, Ironside J, Lucraft H, Gilbertson R, Tait DM, Walker DA, Pizer BL, Imeson J, Lashford LS; International Society of Paediatric Oncology; United Kingdom Children’s Cancer Study Group (2003) Results of a randomized study of preradiation chemotherapy versus radiotherapy alone for nonmetastatic medulloblastoma: The International Society of Paediatric Oncology/United Kingdom Children’s Cancer Study Group PNET-3 Study. J Clin Oncol 21(8):1581–1591PubMedCrossRefGoogle Scholar
  36. 36.
    Taylor RE, Bailey CC, Robinson KJ, Weston CL, Walker DA, Ellison D, Ironside J, Pizer BL, Lashford LS (2005) Outcome for patients with metastatic (M2-3) medulloblastoma treated with SIOP/UKCCSG PNET-3 chemotherapy. Eur J Cancer 41(5):727–734PubMedCrossRefGoogle Scholar
  37. 37.
    Thomas PR, Deutsch M, Kepner JL, Boyett JM, Krischer J, Aronin P, Albright L, Allen JC, Packer RJ, Linggood R, Mulhern R, Stehbens JA, Langston J, Stanley P, Duffner P, Rorke L, Cherlow J, Friedman HS, Finlay JL, Vietti TJ, Kun LE (2000) Low-stage medulloblastoma: final analysis of trial comparing standard-dose with reduced-dose neuraxis irradiation. J Clin Oncol 18(16):3004–3011PubMedGoogle Scholar
  38. 38.
    Weil MD, Lamborn K, Edwards MS, Wara WM (1998) Influence of a child’s sex on medulloblastoma outcome. JAMA 279(18):1474–1476PubMedCrossRefGoogle Scholar
  39. 39.
    Yao MS, Mehta MP, Boyett JM, Li H, Donahue B, Rorke LB, Zeltzer PM (1997) The effect of M-stage on patterns of failure in posterior fossa primitive neuroectodermal tumors treated on CCG-921: a phase III study in a high-risk patient population. Int J Radiat Oncol Biol Phys 38(3):469–476PubMedCrossRefGoogle Scholar
  40. 40.
    Zeltzer PM, Boyett JM, Finlay JL, Albright AL, Rorke LB, Milstein JM, Allen JC, Stevens KR, Stanley P, Li H, Wisoff JH, Geyer JR, McGuire-Cullen P, Stehbens JA, Shurin SB, Packer RJ (1999) Metastasis stage, adjuvant treatment, and residual tumor are prognostic factors for medulloblastoma in children: conclusions from the Children’s Cancer Group 921 randomized phase III study. J Clin Oncol 17(3):832–845PubMedGoogle Scholar

Copyright information

© Springer-Verlag 2006

Authors and Affiliations

  • D. Kombogiorgas
    • 1
  • S. Sgouros
    • 1
  • A. R. Walsh
    • 1
  • A. D. Hockley
    • 1
  • M. Stevens
    • 5
  • R. Grundy
    • 4
  • A. Peet
    • 2
  • M. English
    • 2
  • D. Spooner
    • 3
  1. 1.Department of NeurosurgeryBirmingham Children’s HospitalBirminghamUK
  2. 2.Department of OncologyBirmingham Children’s HospitalBirminghamUK
  3. 3.Department of RadiotherapyBirmingham Children’s HospitalBirminghamUK
  4. 4.Department of OncologyQueen’s Medical CentreNottinghamUK
  5. 5.Department of OncologyBristol Children’s HospitalBristolUK

Personalised recommendations